Thrombotic thrombocytopenic purpura

TTP - this is worth waking up the on call haematologist.
Etiology Autoimmune (40-70%), drug-related (e.g. clopidogrel), hereditary ADAMST13 mutations... see below
Clinical presentation Fever, MAHA, purpuric rash, neuro symptoms, AKI
Complications:
Pathogenesis ADAMTS13 damage or inhibition → vWF not cleaved → microvascular thrombosis
Diagnostic investigations Thrombocytopenia; ↑LDH, ↓ haptoglobins, clotting (normal). Exclude infection. Blood film: schistocytes
Management Plasma exchange, FFP, rituximab

etiologies

  • Autoimmune haemolysis/Evans syndrome
  • Disseminated intravascular coagulation
  • Pregnancy‐associated e.g. HELLP (haemolysis, elevated liver enzymes and low platelets), eclampsia
  • Drugs eg quinine, simvastatin, interferon, Calcineurin inhibitors
  • Malignant hypertension
  • Infections, typically viral (cytomegalovirus, adenovirus, Herpes simplex virus) or severe bacterial (meningococcus, pneumococcus), fungal
  • Autoimmune disease (lupus nephritis, acute scleroderma)
  • Vasculitis
  • Haemolytic uraemic syndrome (diarrhoea positive/negative)
  • Malignancy
  • Catastrophic antiphospholipid syndrome